METHODS: The charts of all
juvenile polyposis syndrome patients who had had at least one colonic operation since 1953 in our institution were reviewed. The following data were abstracted: demographics, the number and site of the
polyps, symptoms, the intervals and types of the colonic operation, follow-up, and the patients' current status.
RESULTS: There were 13 patients (6 males) with a median age of 10 years (range, 1-50 years) at the time of diagnosis. Patients had colonic (n = 13), rectal (n = 12), and gastric (n = 6)
polyps. Rectal
bleeding (n = 11) was the most common presenting symptom. Three patients underwent
proctectomy as the initial operation. Although a rectum-preserving operation was initially performed in ten patients, a subsequent
proctectomy was required in five of them within a median of 9 years (range, 6-34 years). Therefore, eight patients had their rectum removed during the study period; five had an
ileal pouch-anal anastomosis, one had a Koch pouch as a restorative surgery, and two had an end
ileostomy. No relation was observed between the number of colonic and rectal
polyps and the type of surgery or the need for
proctectomy. Patients were followed up a median of 3 years (range, 2-24 years) after their ultimate operations. During this period, one patient (20 percent) who underwent restorative
proctectomy and 4 patients (80 percent) whose rectums were preserved required multiple endoscopic polypectomies for recurrent
polyps in the pouch (first patient) or their rectums (the other four patients). The patient who underwent the Koch procedure required surgery for recurrent
polyps in her pouch.
CONCLUSIONS: One-half of the patients who initially underwent rectal preservation required subsequent
proctectomy. The number of colonic or rectal
polyps does not influence the choice of the
surgical procedure. Both
restorative proctocolectomy and subtotal
colectomy with ileorectal anastomosis need endoscopic follow-up because of the high recurrence rates of juvenile
polyps in the remnant rectum or pouch.